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Refractory Hypoxemia in a 23-Year-Old Patient With Budd-Chiari SyndromeBicaval Syndrome FREE TO VIEW

Jeroen J. H. Bunge, MD; Ubbo S. Wiersema, MD; Adriaan Moelker, MD, PhD; Jasper van Bommel, MD, PhD; Eric T. T. L. Tjwa, MD, PhD
Author and Funding Information

From the Department of Intensive Care (Drs Bunge and van Bommel), the Department of Gastroenterology and Hepatology (Drs Wiersema and Tjwa), and the Department of Radiology (Dr Moelker), Erasmus Medical Centre University Hospital, Rotterdam, The Netherlands.

CORRESPONDENCE TO: Eric T. T. L. Tjwa, MD, PhD, Department of Gastroenterology and Hepatology, Erasmus Medical Centre University Hospital, Room Hs-312, PO Box 2040, 3000 CA Rotterdam, The Netherlands; e-mail: etjwa@erasmusmc.nl


Drs Bunge and Wiersema contributed equally to this work.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(5):e149-e152. doi:10.1378/chest.13-2879
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Antiphospholipid syndrome is an autoimmune disorder characterized by a hypercoagulable state, leading to arterial and venous thrombosis. We present a 23-year-old patient, suspected of having Budd-Chiari syndrome due to antiphospholipid syndrome, who developed severe and progressive hypoxemia, requiring prolonged mechanical ventilation. After a detailed but unsuccessful workup, a contrast CT scan revealed an occluded superior vena cava and azygos vein-superior vena cava junction and massive right-to-left shunting through a network of systemic to pulmonary venous collaterals. Restoring normal blood flow from the azygos vein into the right atrium by stenting the azygos-superior vena cava junction resolved the hypoxemia immediately. Within the same procedure, the hepatic outflow obstruction was successfully treated by stenting a severe stenosis of the suprahepatic inferior vena cava caused by calcified thrombus.

Figures in this Article

Hypercoagulation may be presented by a variety of symptoms, depending on the vasculature affected. In this article, we describe a patient with dyspnea, suspected of having vascular liver disease.

A 23-year-old woman was referred to our hospital for analysis and treatment of a suspected Budd-Chiari syndrome (BCS), with symptoms of ascites and peripheral edema. Furthermore, she complained of progressive dyspnea. Earlier CT scanning had shown a suprahepatic calcified thrombus in the inferior vena cava (IVC) as a possible cause of the hepatic outflow obstruction or BCS. Her medical history reported antiphospholipid syndrome (positive lupus anticoagulans, positive anti-β2 glycoprotein antibodies, and positive cardiolipid IgG, and no known predisposing disorders) complicated by pulmonary emboli, and thrombosis of the superior vena cava (SVC) and brachiocephalic vein 1 year previously, for which she was still using anticoagulants, with international normalized ratio in the target range (2,5-3,5).

On physical examination, she had a respiratory rate of 20 breaths/min and peripheral capillary oxygen saturation of 85% on ambient air. Examination of the head revealed marked venous distension. There were no cardiac murmurs, and breathing sounds were normal. Platypnea was absent. Ascites and pedal edema were present. One day after admission, her condition deteriorated. She was intubated because of progressive hypoxic respiratory failure. With a positive end expiratory pressure of 10 cm H2O and an Fio2 of 0.7, the arterial Po2 was only 60 mm Hg. Recruitment maneuvers did not improve oxygenation. A CT scan, with iodine contrast injected through the femoral vein, showed marginal pleural effusion, atelectasis, and no signs of pulmonary embolism. The liver parenchyma was homogenous, with patent flow in the portal veins. There were ascites without splenomegaly. Stenotic circular calcification in the IVC, just below the right atrium, was noted.

As a next step, in the setting of BCS, portopulmonary hypertension and hepatopulmonary syndrome were excluded as causes of the hypoxemia. Venous catheterization by right femoral access revealed a pressure gradient of 16 mm Hg (25-9 mm Hg) over the IVC stenosis. Pulmonary pressures were not elevated. Contrast echocardiography (using agitated saline injected through the femoral artery) was unremarkable and showed no signs of a right-left shunt. 99mTechnetium macroaggregated albumin perfusion scanning showed 22% macroaggregated albumin capture in the cerebrum/kidney, possibly indicating pulmonary vascular shunting.

Upon further respiratory deterioration, a CT scan was repeated 3 weeks later to rule out new pulmonary embolism. By chance, the iodine contrast was now administered through the antecubital vein in the right arm. This scan showed extensive thrombosis in the SVC, resulting in total occlusion above, and subtotal occlusion below, the level of the azygos ostium. The azygos vein was patent and dilated. There were extensive chest wall and neck collaterals entering the azygos and hemiazygos veins and the right pulmonary veins. There was almost no contrast in the right atrium and the pulmonary arteries, but dense contrast enhancement in the right pulmonary veins, left atrium, and left ventricle, consistent with direct shunting between systemic veins and pulmonary veins based on an SVC syndrome, rather than hepatopulmonary syndrome (Figs 1, 2).

Figure Jump LinkFigure 1 –  CT scan with iodine contrast administered through the right antecubital vein. A, Extensive chest wall collaterals are visible. There is almost no contrast in the right atrium and the pulmonary arteries, but dense contrast enhancement in the right pulmonary veins, left atrium, and left ventricle. B, Lung window.Grahic Jump Location
Figure Jump LinkFigure 2 –  Abnormal collaterals as a result of bicaval obstruction leading to systemic hypoxemia. Ao = aorta; AV = azygos vein; IVC =  inferior vena cava; LA = left atrium; LV = left ventricle; PA = pulmonary artery; PV = pulmonary vein; RA = right atrium; RV = right ventricle; SVC = superior vena cava.Grahic Jump Location

Angiography was performed, using a femoral approach. A balloon expandable stent (Scuba, 8 × 37 mm; Medtronic) was placed to dilate the azygos-SVC junction and was inflated to 10 mm in diameter. In addition, a self-expandable stent (Sinus XL, 25 × 60 mm; Optimed) was placed over the calcified stenosis in the IVC (Fig 3). Oxygenation improved markedly within a minute after stent placement, and the patient was weaned from the ventilator within 24 h. The ascites resolved completely within 2 weeks.

Figure Jump LinkFigure 3 –  A, AV and SVC before stent placement, with occlusion of the SVC. Extensive collaterals originating from the AV are visible. B, AV and SVC after stent placement. Rapid flow of contrast, with no visible collaterals left. C, IVC, with significant stenosis right below the RA. D, IVC after stent placement. See Figure 2 legend for expansion of abbreviations.Grahic Jump Location

We present a patient with antiphospholipid syndrome, resulting in BCS due to IVC thrombus, and progressive hypoxemia due to SVC syndrome, causing a severe systemic to pulmonary veno-venous shunt. Repeated use of the femoral route for contrast administration for CT scanning, echocardiography, and radionuclide imaging showed either no or minor shunting, which may have been part of coexisting hepatopulmonary syndrome. To our knowledge, this combination has never been described in the literature.

Typically, in SVC syndrome, the occluded SVC is bypassed predominantly by the azygos, as well as by the internal mammary, lateral thoracic, and vertebral venous systems.1,2 In this patient, the azygos-SVC junction was occluded subtotally as well. Driven by increased venous pressures, unusual collaterals were established between the systemic and pulmonary veins, resulting in a right-to-left shunt. This phenomenon has been described previously in the literature in SVC syndrome, and several anatomic substrates have been identified.2-5 In this patient, the collaterals were most likely the result of reversed flow through anatomic connections between the azygos veins and the pleurohilar pulmonary and bronchial veins. This is a network of veins, rather than a single connection, making this unsuitable for interventions. In this patient, stenting of the azygos-SVC junction restored venous flow from the well-developed azygos vein into the right atrium, diminishing venous pressures, and resulting in collapse of the systemic to pulmonary venous collaterals.

Other features of SVC are tortuous cutaneous venules and veins on the chest and neck, which could also be seen in this patient.6 Facial edema and swelling of the arms may occur. Other rare manifestations that may occur in SVC syndrome are ischemic stroke by systemic embolism through systemic to pulmonary venous connections, or the development of other unusual collateral networks, such as cavo-portal connections2,7

In conclusion, in this patient, the antiphospholipid syndrome was complicated by bicaval obstruction, resulting in suprahepatic BCS, and hypoxemia due to systemic to pulmonary veno-venous shunting. By stenting the azygos-SVC junction and the IVC stenosis, both conditions were treated adequately.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

BCS

Budd-Chiari syndrome

IVC

inferior vena cava

SVC

superior vena cava

Sheth S, Ebert MD, Fishman EK. Superior vena cava obstruction evaluation with MDCT. AJR Am J Roentgenol. 2010;194(4):W336-W346. [CrossRef] [PubMed]
 
Kapur S, Paik E, Rezaei A, Vu DN. Where there is blood, there is a way: unusual collateral vessels in superior and inferior vena cava obstruction. Radiographics. 2010;30(1):67-78. [CrossRef] [PubMed]
 
Cihangiroglu M, MacEneaney P, MacMahon H, Dachman A. Systemic to pulmonary venous shunt in superior vena cava occlusion. J Thorac Imaging. 2000;15(3):208-210. [CrossRef] [PubMed]
 
Herscovici R, Szyper-Kravitz M, Altman A, et al. Superior vena cava syndrome-- changing etiology in the third millennium. Lupus. 2012;21(1):93-96. [CrossRef] [PubMed]
 
Arrivé L. Systemic-to-pulmonary venous shunt in superior vena cava obstruction. AJR Am J Roentgenol. 2011;197(1):W193. [CrossRef] [PubMed]
 
Hirschmann JV, Raugi GJ. Dermatologic features of the superior vena cava syndrome. Arch Dermatol. 1992;128(7):953-956. [CrossRef] [PubMed]
 
Nascimbene A, Angelini P. Superior vena cava thrombosis and paradoxical embolic stroke due to collateral drainage from the brachiocephalic vein to the left atrium. Tex Heart Inst J. 2011;38(2):170-173. [PubMed]
 

Figures

Figure Jump LinkFigure 1 –  CT scan with iodine contrast administered through the right antecubital vein. A, Extensive chest wall collaterals are visible. There is almost no contrast in the right atrium and the pulmonary arteries, but dense contrast enhancement in the right pulmonary veins, left atrium, and left ventricle. B, Lung window.Grahic Jump Location
Figure Jump LinkFigure 2 –  Abnormal collaterals as a result of bicaval obstruction leading to systemic hypoxemia. Ao = aorta; AV = azygos vein; IVC =  inferior vena cava; LA = left atrium; LV = left ventricle; PA = pulmonary artery; PV = pulmonary vein; RA = right atrium; RV = right ventricle; SVC = superior vena cava.Grahic Jump Location
Figure Jump LinkFigure 3 –  A, AV and SVC before stent placement, with occlusion of the SVC. Extensive collaterals originating from the AV are visible. B, AV and SVC after stent placement. Rapid flow of contrast, with no visible collaterals left. C, IVC, with significant stenosis right below the RA. D, IVC after stent placement. See Figure 2 legend for expansion of abbreviations.Grahic Jump Location

Tables

References

Sheth S, Ebert MD, Fishman EK. Superior vena cava obstruction evaluation with MDCT. AJR Am J Roentgenol. 2010;194(4):W336-W346. [CrossRef] [PubMed]
 
Kapur S, Paik E, Rezaei A, Vu DN. Where there is blood, there is a way: unusual collateral vessels in superior and inferior vena cava obstruction. Radiographics. 2010;30(1):67-78. [CrossRef] [PubMed]
 
Cihangiroglu M, MacEneaney P, MacMahon H, Dachman A. Systemic to pulmonary venous shunt in superior vena cava occlusion. J Thorac Imaging. 2000;15(3):208-210. [CrossRef] [PubMed]
 
Herscovici R, Szyper-Kravitz M, Altman A, et al. Superior vena cava syndrome-- changing etiology in the third millennium. Lupus. 2012;21(1):93-96. [CrossRef] [PubMed]
 
Arrivé L. Systemic-to-pulmonary venous shunt in superior vena cava obstruction. AJR Am J Roentgenol. 2011;197(1):W193. [CrossRef] [PubMed]
 
Hirschmann JV, Raugi GJ. Dermatologic features of the superior vena cava syndrome. Arch Dermatol. 1992;128(7):953-956. [CrossRef] [PubMed]
 
Nascimbene A, Angelini P. Superior vena cava thrombosis and paradoxical embolic stroke due to collateral drainage from the brachiocephalic vein to the left atrium. Tex Heart Inst J. 2011;38(2):170-173. [PubMed]
 
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